Session Information
Date: Monday, June 20, 2016
Session Title: Parkinsonism, MSA, PSP (secondary and parkinsonism-plus)
Session Time: 12:30pm-2:00pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: The aim of the present study is to describe one of the largest series of histologically proven multiple system atrophy (MSA) patients, who underwent deep brain stimulation (DBS) because of an atypical clinical presentation suggestive of Parkinson’s disease (PD).
Background: MSA is a rapidly progressive condition characterized by a levodopa unresponsive Parkinsonian syndrom, associated with dysautonomia, pyramidal tract involvement and cerebellar signs. Some patients with MSA, proven histologically, may present slow and relatively less severe disease progression than usual mimicking PD. In such cases but also in more typical MSA patients, DBS has been proposed and reported mostly as case reports and very few little series.
Methods: The present study describes one of the largest series of histologically proven MSA patients, who underwent DBS because of an atypical clinical presentation suggestive of PD and who dramatically worsened after surgery. Five histologically proven MSA patients, who had underwent DBS were identified retrospectively in the French MSA database.
Results: The evolution of each of these patients was disastrous and death occurred quickly after surgery despite a long disease duration before surgery, which indicates a probable deleterious effect of DBS on disease course. Another interesting point of the present series is the atypical presentation of these patients. Indeed all these patients were misdiagnosed as having PD because of the absence of red flags such as pyramidal tract involvement, ataxia, marked dysautonomia (except one patient presenting isolated atypical dyspnea and another one urinary dysfunction and impotenza) even after 5 years disease progression
| Patients | Age at onset (years) | Disease duration (years) | Red flags | Delay between disease onset and DBS (years) | Target | Motor improvement after levodopa challenge | Outcome after surgery |
| 1 | 55 | 15 | Dyspnea revealing a dysautonomia | 8 | NST | 59% | No motor improvement/Falls, urinary incontinence : 1 year after DBS/Bedridden 5 years after DBS/Death 7 years after DBS |
| 2 | 64 | 10 | Facial Dystonia | 7 | NST | 80% | Motor improvement during 1 year/ Falls, dysarthria, dysphagia, cognitive decline 1 year after DBS/ Death 4 years after DBS |
| 3 | 57 | 7 | Dysuria, early hypophonia | 5 | NST | 49% | No motor improvement after DBS/ Death 2 years after DBS, after a surgery for prostatic adenoma |
| 4 | 40 | 18 | Dysuria and impotenza | 8 | NST | 73% | No motor improvement after DBS/ Falls, ataxia, dysarthria immediately after DBS/ Death 7 years after DBS |
| 5 | 55 | 7 | None | 6 | NST | 88% | No motor improvement/ Falls, dysarthria, ataxia after DBS/ Death 1 year after DBS |
Conclusions: This study, in light with previous reports demonstrate that DBS is ineffective in MSA and may on the contrary lead to a major worsening of clinical presentation and ultimately to death.
To cite this abstract in AMA style:
C. Laurencin, W. Meissner, C. Tranchant, T. Witjas, F. Viallet, P. Damier, T. Danaila, J.L. Houeto, A. Eusebio, S. Thobois. Deep brain stimulation may lead to dramatic worsening of slowly progressive multiple system atrophy: A clinico-pathological series and review of the literature [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/deep-brain-stimulation-may-lead-to-dramatic-worsening-of-slowly-progressive-multiple-system-atrophy-a-clinico-pathological-series-and-review-of-the-literature/. Accessed November 22, 2024.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-may-lead-to-dramatic-worsening-of-slowly-progressive-multiple-system-atrophy-a-clinico-pathological-series-and-review-of-the-literature/